Sickle-cell Anemia

March 4, 2010 by admin  
Filed under General Law

Sickle-cell anemia (or sickle-cell disease) is a genetic blood disorder which is characterized by the individual’s red blood cells (RBCs) assuming a rigid, abnormal, curved sickle shape. This will decrease the RBC’s flexibility, thus causing a variety of medical complications. This abnormal shape of the RBCs occurs as a result of mutation in the hemoglobin gene. Prognosis for this disease includes a shortened life span, with the average life expectancy of 42 years for males and 48 years for females.

This disease will usually present in early childhood and is far more common for those who are descendants of people living in tropical and sub-tropical regions where malaria is/was common. The reason for this has to do with a higher survival rate among those who carry a single sickle-cell gene. As such, approximately 1/3 of the indigenous population of the Sub-Sahara region of Africa carries the sickle-cell gene. Those who carry only one of the sickle-cell genes are more immune to malaria, since the infestation of malaria plasmodium is halted by the blood cell’s sickling.

In the United States, approximately 1 in 5,000 residents have the sickle-cell trait (mostly descendants of Sub-Saharan Africans) with 1 in 500 African American births possessing the disease.

Those infected can demonstrate a variety of symptoms to include; anemia, Sequestration Crisis Aplastic Crisis, Hyper Hemolytic Crisis and others. Most of these crises will last between five and seven days. The spleen can also be affected because of its narrow blood vessels and its function of eliminating defective blood cells. The spleen will usually be infarcted before the end of the patient’s childhood and this will increase the risk of infection from encapsulated organisms, due to the autosplenectomy. Because of this, vaccinations and antibiotics will be prescribed.

One of the earliest clinical signs of sickle-cell is inflammation of the fingers and/or toes (dactylis) which can be quite painful and can occur as early as 6 months of age, lasting for up to a month. Another symptom is acute chest syndrome which is characterized by fever, breathing difficulties and chest pain. Usually, the patient will be treated for both sickling and pneumonia. In some situations, surgery may also be indicated.

Please note that this article is for informational purposes only and is not intended as legal advice.

Sickle-cell disease Social Security disability claim information may be obtained by visiting the website of Fort Worth SSI Attorney Gerard Lynch, representing Social Security cases throughout Texas.

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